A patient with Von Hippel disease is described. The Von Hippel syndrome is a genetic disease characterized by a retinal angiomatosis and multiple tumors (renal and pancreatic sites are common). The pheochromocytoma is-on the contrary-very uncommon and the association with 2 pheochromocytomas has probably never been reported. In our case, a double pheochromocytoma (the first in 1973, the second in 1986) was present, in conjunction with a thyroid tumor and with an anomalous renal vein (circumaortic ring). The clinical picture was characterized by a severe hypertension and a fourth stage retinopathy. A correct diagnosis was established only 10 years after the clinical exordium. The thyroidectomy and the ablation of both the pheochromocytomas were necessary.
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